Presentation
3,6) The rarest pattern--and that most representative of an advanced disease state--is uremic leontiasis ossea which presents as marked expansion of the mandible and maxilla, occasionally with obliteration of the maxillary sinuses and splaying of the [freethesaurus.com]
Herein, we present a 31-year-old man diagnosed with uremic leontiasis ossea. [cureus.com]
This study presents a report on a patient with advanced hyperparathyroidism with skeletal changes compatible with leontiasis ossea. [journalrip.com]
[…] characteristic radiologic features of uremic leontiasis ossea. 6 Case Report We present case report of two patients (12-year-old girl and 29- year-old male) presenting with jaw masses. [slideplayer.com]
Entities such as cherubism usually present at 2-3 years of age and spontaneously resolve after puberty. 8 Paget’s disease typically presents later than the current patient’s age and has a male predilection. [appliedradiology.com]
Entire Body System
- Dentist
The book will appeal to clinical geneticists, pediatricians, neurologists, head and neck surgeons, otolarynologists, and dentists. The 4th edition, which published in 2001, has sold 2,600 copies. [books.google.com]
Spec Care Dentist. 2003;1:28-34. Yang Q et al. Skeletal Lesions in primary hyperparathyroidism. Am J Med Sci. 2015;349(4):321-327. Cure JK, Vattoth S, Shah R. Radiopaque jaw lesions: an approach to the differential diagnosis. [appliedradiology.com]
Spec Care Dentist. 2018, 38:163-171. 10.1111/scd.12284 Duan SY, Xing CY, Yang G, Wang NN, Zhang B: Dramatic alteration of the skull in a uremic patient with leontiasis ossea. [cureus.com]
- Noncompliance
Since childhood, she had been noncompliant with her diet and medication; her secondary hyperparathyroidism deteriorated over the years. [ncbi.nlm.nih.gov]
- Congestive Heart Failure
heart failure due to shunting of blood through warm skin (bone is hypervascular and hot) Leontiasis ossea : cranium too heavy to lift Platybasia : invagination of base of skull due to weak bone, compression of posterior fossa structures Associated neoplasms [pathologyoutlines.com]
- Feeding Difficulties
difficulties.[1] When this occurs, it is called uremic leontiasis ossea and has characteristic imaging findings. [clinicalimagingscience.org]
Respiratoric
- Orthopnea
He also complained of shortness of breath with exertion, orthopnea, as well as mild chest pain. Fluid overload was suspected due to the patient's missed dialysis. [cureus.com]
- Cough
He denied any headaches, fever, cough, nausea, vomiting or naso-oropharyngeal discharge. As per patient's report, he had a similar presentation affecting his left jaw in 2012. [cureus.com]
Cardiovascular
- Heart Failure
failure due to shunting of blood through warm skin (bone is hypervascular and hot) Leontiasis ossea : cranium too heavy to lift Platybasia : invagination of base of skull due to weak bone, compression of posterior fossa structures Associated neoplasms [pathologyoutlines.com]
There was also documentation supporting severe cardiovascular disease resulting in heart failure and diabetes mellitus. [clinicalimagingscience.org]
- Chest Pain
He also complained of shortness of breath with exertion, orthopnea, as well as mild chest pain. Fluid overload was suspected due to the patient's missed dialysis. [cureus.com]
Jaw & Teeth
- Jaw Pain
Facial swelling and bilateral or unilateral jaw pain may be part of the initial complaints. [cureus.com]
Neurologic
- Stroke
Involvement of the carotid canal at the skull base if severe enough could result in progressive compression of the passing internal carotid artery and potentially increase the risk of stroke. [clinicalimagingscience.org]
Workup
X-Ray
- Rugger-Jersey Spine
When described, it is termed a rugger jersey spine given it close resemblance to the pattern seen on a rugby jersey. [clinicalimagingscience.org]
Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior [file.scirp.org]
Treatment
It is possible that at this stage of the disease, the patient has tertiary hyperparathyroidism when treatment is rarely successful7. [ncbi.nlm.nih.gov]
A medical practitioner should always be consulted for diagnosis and treatment of all medical conditions. [hospitalhelp.co.uk]
Review the unique radiologic findings, differential diagnosis, treatment and follow up in uremic leontiasis ossea. [posterng.netkey.at]
HD treatment was initiated and further post-HD evaluation showed improvement of the patient's overall status. [cureus.com]
Prognosis
More often, most lesions can be managed through staged procedures with overall very favorable outcomes and good long term prognosis. [skullbaseinstitute.com]
Treatment usually entails parathyroidectomy with monitoring of serum calcium, phosphate, and vitamin D levels while on appropriate replacement (calcium/vitamin D) or binding (phosphate) pharmacotherapy. 4 The prognosis is usually favorable. [appliedradiology.com]
Etiology
Clinical-Medical Image: Paget disease of the bone is a chronic disorder of unknown etiology that typically results in enlarged, deformed bones due to abnormal and excessive bone remodeling. [imagejournals.org]
Indian J Ophthalmol 1969;17:166-70 Introduction The term leontiasis ossea was first coined by Virchow [6] in 1896 to describe the leonine appearance of the facial deformity in this rare condition of unknown etiology. [ijo.in]
Case Presentation A 31-year-old man with a past medical history of ESRD of unknown etiology, on hemodialysis (HD) since 2004, presented to the emergency department (ED) complaining of missed dialysis. [cureus.com]
However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5]. After parathyroidectomy, facial changes can be stabilized or improve mildly. [file.scirp.org]
Epidemiology
The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. [books.google.com]
Pathophysiology
The report aims to review the pathophysiology of the condition as described in the literature, the patient presentation and imaging modalities used to investigate, and classical findings seen in patients with uremic leontiasis ossea. [cureus.com]
Pathophysiological origin of the disease is not clearly understood. However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5]. [file.scirp.org]
Prevention
Strict medical management of metabolic derangements, prevention of secondary hyperparathyroidism and adherence to hemodialysis are the best possible options to prevent this condition. [cureus.com]
Hence, early recognition and treatment of leontiasis ossea can prevent its complications. Please cite this paper as: Raffiei Jelodar H, Yarmohamadi M, Baghai Wadji M, Babaei MR. Leontiasis ossea; a rare presentation of secondary hyperparathyroidism. [journalrip.com]
Early recognition andconfident diagnosisof ULO is essential to prevent progression to facial disfigurement andavoid invasive procedures. [posterng.netkey.at]
During hemodialysis patients often develop numerous comorbid conditions that may interfere with the existing disease and prevent further treatment, as was the reported case. [ncbi.nlm.nih.gov]